CTICasesNeurofibromatosis Type 1 (NF1) with Pulmonary Cysts and Malignant Peripheral Nerve Sheath Tumor (MPNST)
Imaging findings
Chest CT shows a few small, scattered, thin-walled pulmonary cysts in a random distribution. Soft-tissue windows reveal multiple subcutaneous and chest wall nodules consistent with cutaneous or plexiform neurofibromas. The patient also has a history of a malignant peripheral nerve sheath tumor (sarcoma) in the hand.
Key takeaways
Neurofibromatosis type 1 (NF1) can be associated with diffuse, small, thin-walled pulmonary cysts, which are usually sparse and located predominantly in the upper or subpleural zones. NF1 patients are also at increased risk for malignant peripheral nerve sheath tumors (MPNST), which can arise from pre-existing neurofibromas.
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